| The Background and Objective:The vasculitis is a diverse group of systemic or localised disorders in which blood vessel walls are infiltrated and damaged by inflammatory cells, which can cause secondary injuries of many organs.The vasculitic neuropathy(VNs) is a group of disorders characterized by ischaemic injury of peripheral nerves due to the inflammatory destruction of nerve blood vessels which caused by vasculisis. Vasculitis can be primary or secondary to many other diseases(such as rheumatic disease,infection or diabetes mellitus).Vasculitic neuropathy can be categorised mainly into primary systemic vasculitides, secondary systemic vasculitides, and nonsystemic or localised vasculitis. As a group of hetero-geneous dis-orders, vasculitis make its diagnosis and treatment difficult for the physicians due to its low incidence and complicated clinical features,they may be vital without timely diagnosis and treat-ment.In this research, the clinical and pathological features of 28 cases of VNs which are diagnosed and treated at Qilu Hospital in 1999-2016 were retrospectively reviewed. The patients were followed-up and course were recorded and analysed. The main objective of this study is to summarize the clinical and pathological features of VNs, to conduct the follow-up study of the patients, and to discuss the factors affecting the prognosis, which may provide a reference for the diagnosis and treatment of VNsMaterial and methods:All the 28 cases were diagnosed with VNs by the research institute of neuromascular and neurodegenerative disease of SDU in 1999.1-2016.2. Peripheral nerve biopsy was performed in 28 patients, of which 3 patients underwent muscle biopsy. The HE/MGT/ toluidine blue staining and immunohistochemical staining were performed on transverse nerve sections,the HE/MGT/NADH/SDH/COX/ATPase staining and immunohistochemical staining were performed on transverse muscle sections. The medical records of the patients were obtained from the record room of Qilu Hospital. The course of these patients and their outcomes were achieved through 1-8 years follow-up by telephone. Then we summarize and discuss the clinical features, courses and outcomes of the patients.Outcomes:Of the 28 patients,13 were female and 15 male. Patients aged 20-71 years old, the average age is (51.12+12.13) years old. The average time interval between onset to diagnosis is (11.6+20) month. The initial symptom of 12cases(41%) were mononeuropathy,10 cases(36%) were symmetrical polyneuropathy of lower limbs,6 cases(21%)were symmetrical polynueropathy of four limbs. Until the diagnosis, The upper and lower limbs were involved in 19 cases (68%),9 patients were diagnosed with only the upper limb or lower limb (32%). For the motor impairment, proximal muscles were worse than distal muscles in 14cases (50%), milder in 3 cases,both are similar in 7cases(21%), distal impaired only in 2 cases,2 cases had no impair-ment.There are 7 cases with anemia,18 cases with ESR increased,14 cases with CRP positive,6 cases with ANCA positive,4 cases with ANA positive,9 cases with RF positive. 3 cases with eosinophilia,4 cases had incresed protein in urine,2 cases had increased RBC count in urine.23 cases of patients had electrophysiological examination, which showed 18 cases with axonal damage,5 cases with axonal damage and demyelination. Nerve biopsy showed a reduction in myelinated fiber density. Transmural inflammatory cell infiltration of vessels in epineurium and perineurium were seen in 22 cases,6 cases had perivasculisis only.3 cases which had muscle biopsy showed neurogenic impairment, and vasculisis was found.In 28 patients pathologically diagnosed with VNs,16 patients were diagnosed with systemic vasculitis neuropathy (SVN),12 patients systemic vasculitic neuropathy (NSVN). In SVN patients,8 cases were diagnosed with ANCA associated vasculitis neuropathy (AVV),4 cases were secondary to rheumatoid arthritis in,1 cases was secondary to SLE, and another 2 patients can not be diagnosed definitely.All the 28 patients were followed-up for 2 weeks-9 years, (25.6±31.1)months in average. After treatments 1 patients achieved remission,two of them relapsed due to fatigue or fever and achie-ved remission again after treatment.3 patients had improved to some degree in hospital then aggravated after discharge.they died of dyspnea.2 patients didn’t response to therapy and aggravated to death. Tow patients who refused immunosuppressive therapy neither improved nor aggravated.Conclusions:1. SVNs is more common than NSVN in this study, AAV is more common type of SVNs.2. Most cases had acute or subacute onset and some patients had insidious onset and slow progression for many years. The majority of patients had initial symptoms such as numbness and weakness of one-side lower or upper limb, but some patients had bilateral dysfunctions at the onset. Nerve involvement mode is mainly multiple mononeuropathy, but most patients were not typical, and showed overlapping multiple mononeuropathy (asymmetric polyneuropathy).3. Until the diagnosis, both upper and lower limbs were involved and both sensory and motor dysfunctions existed for most patients in different degrees. Fever is a common symptom, more common in patients with SVN.4. The laboratory tests showed anemia, thrombocythemia, eosinophilia, increased ESR, etc.. Most NSVN patients showed normal immunologic examinations, but ANA can be positive in some patients. ANCA is essential to the diagnosis of AAV, the negative results cannot exclude the diagnosis. Electromyography mainly showed axonal damage, sometimes combined with the demyelination. Nerve biopsy showed inflammatory infiltration and acute or chronic injury of the vessel walls and decreased myelinated nerves to various degrees.5. The main regimen is the combination of GC and CTX. The majority of patients responsed well to therapy, recurrence is not common. The prognosis of SVN patients is worse than that of NSVN patients. Respiratory system involvement (dyspnea, auscultatory crackles, etc.) indicate an ominous prognosis, the cause of death was respiratory failure and infection. The prevention of infection and hypoproteinemia deserve should be given attention. |
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